Cirrhosis
Epidemiology
Pathophysiology
Presentation
Management
Education
Leading cause of death for >40 yo
Chronic alcohol abuse & viral hepatitis leading causes
Alcoholic cirrhosis most common type in U.S.
Primary biliary cirrhosis (PBS) effecting women ages 40-60
Autoimmune disorders leading to PBS: scleroderma, Raynaud's, thyroid disease, celiac disease, Sjogren's
Primary sclerosis cholangitis (PCS) common in men aged 20-40 associated with IBS
Budd-Chiari Syndrome (BCS) results from hepatic vein thrombosis and outflow obstruction
Untreated Wilson's disease and hemochromatosis are auto recessive metabolic disorders that present with hepatocellular dysfunction
Irreversible end stage liver injury
Chronic inflammation caused by obstruction leading to fibrotic scarring and hepatocellular changes
Causes: Drug induced, Hepatitis B and C, PBC, PSC, autoimmune disorders, hereditary hemochromatosis, vascular disorders
Diffuse disorganization from proliferation of fibrous tissue and nodular regeneration of hepatocytes
Decrease in total liver cell mass due to collagen formation (fibrosis)
Increased resistance to blood flow causing portal venous hypertension
Histological classification: Micronodular/Laennec's (nodules 1 cm or less) or Macronodular (nodules 5 cm)
Typically asymptomatic with enlarged liver or elevated liver enzymes
Weakness, anorexia, weight loss, fatigue, abdominal pain
Esophageal varices, hemorrhoids, or ascites from portal hypertension
Ascites and/or encephalopathy if liver fails
Menstrual abnormalities, loss of libido, impotence, sterility, gynecomastia
Enlarged liver or small in advanced stage
Spider nevi over anterior chest, pectoral alopecia, muscle wasting, Dupuytren's contractions, parotid gland enlargement, palmar erythema, hair loss, testicular atrophy, Caput medusa around umbilical area
glossitis, cheilitis, peripheral neuropathies from vitamin and mineral disturbances
Jaundiced sclera, skin, mucous membranes in later stages
Hyperbilirubinemia
Hematemesis, hematochezia and/or melon from esophageal varices
Hepatic encephalopathy from increasing blood ammonia
Wilson's disease may have golden brown rings of color- Kayser-Fleischer rings located within Descemet's membrane of the cornea
Diagnostics
CBC may show anemia or pancytopenia
PT increases as liver fails
ALT & AST may increase
Gamma-glutamyl transpeptidase level measures recent alcohol ingestion
Alkaline phosphatase increase with binary obstruction
Hypoalbuminemia
Serum bilirubin elevated
Liver biopsy
Abdominal ultrasound to determine size of liver
Doppler to evaluate potency of venous system
CT and MRI to characterize nodules suspicious of malignancies
Liver Changes
diffuse disorganization
bands of fibrous tissue and nodular regeneration of surviving hepatocytes
extent is dependent on the amount of damage done, length of injury, and liver's reaction
decrease in total liver cell mass due to collagen formation
repair process leads to portal venous hypertension
vessels are less efficient than other vessels leading to portal hypertension
Alcohol Induced Liver Disease: abstinence from alcohol
remove causative agents, treat symptoms, and prevent complications
increase 2 year survival rate by 95% if abstain
ensure adequate nutrition
Referrals
hepatologist
nutritionist
protein: 1-1.5 grams per kilogram of body weight per day
if they continue to show clinical decline: liver transplantation
Liver transplant: treatment of choice for irreversible chronic liver disease.
Pharmacotherapeutics
vitamin and mineral supplementation
multivitamin
vitamin B12
thiamine
folate
magnesium
zinc
pharmacotherapeutics post transplant
T-cell depleting monoclonal antibodies (Alemtuzumab)
calcineurin inhibitors
sirolimus
interleukin-2 receptor antagonists
Primary Biliary Cirrhosis: treatment is symptomatic
Cholestyramine: treatment of itching by lowering serum bile acids and increasing the intestinal secretion of bile by preventing its resorption (4-5g TID)
Rifmapin
Ondansetron
replace Vitamin A, D, E, & K orally.
bisphosphonates: treat osteoporosis with bone remodeling associated with PBC.
Calcium supplementation
Ursodiol: stimulates excretion of bile by the liver.
Surgical: reconstruction of biliary tract or liver transplantation
monitor weight daily: monitor increase fluid retention and ascites
promote psychological well being because it is a life threatening terminal diseaes
psychologist
medications that can cause hepatotoxicity such as acetaminophen, Vitamin A, cocaine, tetracycline, phenytoin
avoid CNS depressants: lead to further lethargy and fatigue
enema: self administration if they become constipated or need to decrease the time for bowel resorption
sign/symptoms of infection: fever, pain, chills, body aches, malaise. this can indicate peritonitis.
Complications:
portal hypertension & variceal hemorrhage
disruption of hepatocellular circulation causing an increase in venous pressure
ascites
Treatment of Varices: band ligation and octreotide
Insertion of Blackmore tube for balloon tamponade of varices
Shunt: surgical treatment
excess accumulation of serous fluid within the peritoneal cavity
increased hydrostatic pressure, decreased oncotic pressure, peripheral vasodilation, and volume expansion
1,000mL accumulates within the abdomen
Treatment
Sodium restriction: 400-800mg/day
daily weight monitoring
serum electrolytes
renal function monitoring
Diuretic therapy: Initial - spirinolactone
If diuretic failure and sodium restriction failure - large volume paracentesis is performed
Bacterial Peritonitis
abdominal pain, increasing ascites, fever, progressive encephalopathy
Diagnosed with white count: >300cell/mcL from paracentesis
Gram-negative bacilli: E. coli
Treatment:
cefotaxime: 3rd generation cephalosporin
Norfloxacin: post-SBP prophylaxis
Hepatorenal Syndrome:
oliguria
hyponatremia
azotemia
low urine sodium
hypotension
Hallmark Diagnostic: disproportionate rise in creatinine with respect to BUN
Treatment:
restore intravascular volume: avoid paracentesis and aggressive treatment with diuretics as it can cause too large of a fluid shift
Liver transplantation
Hepatic Encephalopathy
change in mental status resulting from failure of liver to detoxify elements of gut
nitrogenous agents (ammonia) enter the CNS and cause disturbances in neurological system
PE findings:
altered mental status, neuromuscular dysfunction, hyperreflexia
fetor hepaticus
hyperthermia
hyperventilation
Treatment Goal: reduce ammonia formation and other nitrogenous compounds
Iron Deficiency Anemia
Hepatopulmonary Syndrome